All issues > Volume 43(3); 2000

Case Report

J Korean Pediatr Soc. 2000;43(3):444-449. Published online March 15, 2000.

A Month-Old Infant with Chronic Granulomatous Disease Presented as Pulmonary Aspergillosis

SY SY Bae¹, JH JH Jung¹, KH KH Kim¹, YM YM Hong¹, HS HS Koo², JY JY Seoh³, KS KS Hong⁴, ES ES Kang⁴, YS YS Won⁵, JH JH Yoo⁶

¹Departments of Pediatrics, College of Medicine, Ewha Womans University, Seoul, Korea
²Departments of Pathology, College of Medicine, Ewha Womans University, Seoul, Korea
³Departments of Microbiology, College of Medicine, Ewha Womans University, Seoul, Korea
⁴Departments of Clinical Pathology, College of Medicine, Ewha Womans University, Seoul, Korea
⁵Departments of Thoracic Surgery, College of Medicine, Ewha Womans University, Seoul, Korea
⁶Departments of Radiology, College of Medicine, Ewha Womans University, Seoul, Korea

Abstract

Chronic granulomatous disease(CGD) is an uncommon inherited disorder in which phagocytic cells fails to produce antimicrobial superoxide(O2-) due to NADPH oxidase deficiency. This disease can be easily screened by PMA(phobol myristate acetate)-activated nitroblue tetrazolium(NBT) test. Also the disease has features of chronic pyogenic infection, granuloma formation, anemia, hypergammaglobulinemia, and leukocytosis. Treatment uses antibiotics sensitive to specific organism, surgical drainage of abscess and antifungal drugs for fungal infection. Recently, interferon-γ(INF-γ) has been used as treatment and prevention. We experienced a case of CGD with pulmonary aspergillosis in a month-old infant. It was confirmed by activated NBT test. Aspergillus was observed in his lung tissue and Aspergillus fumigatus was cultured from pus of the chest wall. His symptoms improved after antifungal and INF-γ treatment.

Keywords :Chronic granulomatous disease, Pulmonary aspergillosis, INF-γ, Activated NBT test